Wechsler R L, Thiel D V
Am J Dig Dis. 1976 Dec;21(12):1058-69. doi: 10.1007/BF01071863.
This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.
希拉·谢洛克教授对这例复杂的肝胆系统和肾脏纤维多囊病进行了精湛且深刻的分析。她能够区分先天性肝纤维化、卡罗利病以及肝肾成人多囊病，这彰显了她的临床敏锐度。该病例有趣的组织学特征包括肝纤维化伴完整的界板和中央静脉，以及胆管内存在胆栓，但实质内无胆汁淤积。经皮肝穿刺胆管造影显示肝内和肝外胆管扩张。在这类病例中，通过T管引流清除胆道“污垢”有很大局限性。因此，阿德森医生建议经肝放置导管对胆管系统进行冲洗，并施行广泛的胆总管空肠吻合术。谢洛克医生对这种手术方法提出了质疑。有人建议使用鹅去氧胆酸来处理这种“污垢”。尽管胆管扩张且肝脏有病理改变，但患者未出现黄疸，胆道也没有结石。讨论了该患者问题的遗传学情况。
